Background: The involvement of certain organs such as the adrenal gland and ovaries is rare in non-Hodgkin’s lymphoma (NHL). There are few studies comparing clinical features and prognosis based on the extranodal organ involved. Methods: We selected patients presenting with predominantly extranodal involvement among patients diagnosed with NHL from 1998 to 2009 at the Samsung Medical Center. Results: Forty-eight patients with NHL involving rare extranodal sites were analyzed. The extranodal sites were as follows: adrenal gland (n = 14), ovary (n = 13), pancreas (n = 11), uterus (n = 4), esophagus (n = 4) and prostate (n = 2). Diffuse large B-cell lymphoma (DLBCL) was the most common (n = 39), and the median overall survival (OS) was 16.63 months. There was no significant difference in OS according to the involved sites. Rituximab plus CHOP failed to provide an additive survival benefit over CHOP alone in 39 DLBCL patients. The OS of DLBCL in rare extranodal sites was worse than that in common sites when compared based on tumor stage. Conclusions: NHL involving rare extranodal sites had a poor prognosis, and the impact of rituximab on survival was negligible. Thus, more intensive therapeutic strategies should be considered for NHL involving rare extranodal sites.

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